NR AYCU
AU Murayama,S.; Saito,Y.; Hatsuta,H.; Sakiyama,Y.
TI [Neuropathological diagnosis of prion disease]
QU Nippon Rinsho. Japanese Journal of Clinical Medicine 2007 Aug; 65(8): 1401-6
PT english abstract; journal article; review
AB Neuropathological diagnosis of prion disease consists of sequence analysis of PRNP (prion protein gene), located on chromosome 20 and characterization and visualization of deposited proteinase K-resistant prion protein (PrPsc). SNP at 129 locus (M/V) and Type 1 and Type 2 difference in Western blot analysis of PrPsc from the postmortem brain influence the clinical and pathological presentations. Prion disease is classified into sporadic, hereditary and infectious subtypes, but PrPsc from almost all the subtypes can transmit the disease to transgenic mice expressing human PRNP. Variant CJD, apparently derived from bovine spongiformic encephalopathy, requires shift in disease control strategy, in that PrPsc is present in peripheral lymphatic organs.
ZR 6
MH Animals; Brain/metabolism/pathology; Cattle; Chromosomes, Human, Pair 20/genetics; Creutzfeldt-Jakob Syndrome; Humans; Mice; Mice, Transgenic; Polymorphism, Single Nucleotide; PrPsc Proteins/genetics/metabolism; Prion Diseases/classification/*diagnosis/*pathology/transmission; Prions/genetics
AD Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology and Tokyo Metropolitan Geriatric Hospital.
SP japanisch
PO Japan